Stephen A. Paget, MD, FACP, FACR


July 10, 2003


What are the considerations in treating a patient who has both Sjogren's syndrome and primary biliary cirrhosis?

Response from Stephen A. Paget, MD, FACP, FACR

Primary biliary cirrhosis (PBC) is one of the hepatic diseases that is most frequently associated with Sjogren's syndrome (SS), an autoimmune condition characterized by sicca complex (dry eyes, dry mouth). Between 45% and 90% of patients with PBC can exhibit evidence of sialoadenitis (the 2 together are known as 2SS-PBC). However, sicca symptoms severe enough to warrant treatment (ie, eye drops, artificial saliva, pilocarpine drugs that stimulate salivary flow) are seen in a substantially lower frequency, approximately 10%. In addition to the features of PBC (jaundice, hepatomegaly, fatigue, xanthoma, and pruritis) and SS (dry eyes and mouth), pulmonary disease manifested by fever, cough, and dyspnea has been reported. Interstitial lung disease, pulmonary nodules, and granulomas of the lung can be seen. One case of 2SS-PBC associated with mixed-type autoimmune hemolytic anemia has been reported in the literature.[1] Associations with other autoimmune diseases such as thyroid disease, particularly hypothyroidism, have been noted and have a highly significant association with the presence of lacrimal gland dysfunction.[2] Sjogren's syndrome can be associated with extrahepatic and extrasalivary organ dysfunction (such as vasculitis, nephritis, interstitial pneumonitis), and at times these may need to be treated with steroids and/or other immunosuppressive drugs. Malignancy is seen at greater frequency in patients with PBC, most notably breast and primary hepatocellular cancer. Those patients with SS are at increased risk for lymphoproliferative disease.

Antimitochondrial antibodies (AMA) are common in PBC and have been reported in saliva of these patients as well, and may explain the connection between PBC and SS.[3] Autoimmunity is believed to target ductal epithelial cells in diverse organ systems. Antigens have been described that are specifically targeted in the bile duct in PBC. These are expressed aberrantly in salivary glands in as many as 60% of patients with PBC.[4] Autoantibodies to anti-SSB/La are seen in patients with 2SS-PBC, but in lower frequencies (approximately 60% in primary SS vs 40% in PBC). Anti-SSA/Ro antibodies are very common in primary SS, as high as 70%, but are very rarely seen in PBC, in contrast.

Treatment for PBC is commonly ursodeoxycholic acid.[5] While this medication often results in improvement in the liver disease, it does not seem to influence the development or resolution of the associated autoimmune features, such as Sjogren's syndrome. Immunosuppressive agents are not effective in the treatment of PBC, and steroids should be avoided as much as possible because of the disease-related propensity for these patients to develop osteoporosis. Patients should be treated with calcium supplements and bisphosphonates.


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